First Author: S.Khochtali TUNISIA
Co Author(s): I. Ksiaa R. Kahloun J. Nemria S. Ben Yahia S. Zaouali M. Khairallah 0 0 0 0 0 0 0 0 0
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To describe indocyanine angiography (ICGA) findings in hereditary hemorrhagic telangiectasia (HHT) syndrome in two unrelated patients.
Department of Ophthalmology, Fattouma Bourguiba University Hospital, Monastir, Tunisia.
Retropective review of the charts of two male patients, aged 54 and 27 years respectively, with a known history of systemic HHT. The first patient reported left-sided vision decrease. Visual acuity was 20/100. Fundus examination and fluorescein angiography showed shallow extensive infero-temporal exudative retinal detachment with retinal neovascularization in the left eye. The second patient had cerebral vascular accident, with quadranopsia as sequela. Ophtalmological examination and fluorescein angiography results were normal. ICGA was performed in both cases.
In both patients, ICGA revealed bilateral dilatation and tortuosity of choroidal vessels , which were segmental and multifocal. The ectatic portions of choroidal vessels exhibited intense early hyperfluorescence, with no leakage. Lesions were located in the peripheral retina and the posterior pole.
HHT syndrome is a rare disease, with little ocular manifestations except for conjonctival telangiectasia. ICGA may disclose multifocal dilatations. These abnormalities may be asymptomatic or associated with vision loss.